Immunoglobulin prophylaxis in 350 adults with IgG subclass deficiency and recurrent respiratory tract infections: A long-term follow-up

Olinder-Nielsen, A M; Granert, Carl; Forsberg, Pia; Friman, Vanda; Vietorisz, A; Björkander, Janne

doi:10.1080/00365540600951192

Cited by 63 publications

(48 citation statements)

References 16 publications

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“…IgG subclass deficiency is not an indication per se for immunoglobulin replacement, but IgG3 deficiency may be considered a supportive marker for an immune defect [60,61]. In the context of a total IgG < 5 g/l, this will usually mean a reduction of IgG1 as well.…”

Section: Discussionmentioning

confidence: 99%

“…• Concomitant deficiency or reduction of IgA (<0·8 g/l) and/or IgM (<0·4 g/l) [4,56] • Presence of B cells but reduced memory B cell subsets and/or increased CD21 low subsets by flow cytometry [59] • IgG3 deficiency (<0·2 g/l) [60,61] • Impaired vaccine responses compared to age-matched controls • Transient responses to vaccines compared to age-matched controls [62] • Absent isohaemagglutinins (if not blood group AB) [32] • Serological support for autoimmunity in section B, e.g. positive Coombs' test • Sequence variations of genes predisposing to CVID, e.g.…”

Section: Proposed Definition Of Common Variable Immune Deficiency (mentioning

confidence: 99%

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New diagnostic criteria for common variable immune deficiency (CVID), which may assist with decisions to treat with intravenous or subcutaneous immunoglobulin

Ameratunga

Woon

Gillis

et al. 2013

Clinical and Experimental Immunology

197

196

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SummaryCommon variable immune deficiency (CVID) is the most frequent symptomatic primary immune deficiency in adults. The standard of care is intravenous immunoglobulin (IVIG) or subcutaneous immunoglobulin (scIG) therapy. The cause of CVID is currently unknown, and there is no universally accepted definition of CVID. This creates problems in determining which patients will benefit from IVIG/scIG treatment. In this paper, we review the difficulties with the commonly used European Society of Immune Deficiencies (ESID) and the Pan American Group for Immune Deficiency (PAGID) definition of CVID. We propose new criteria for the diagnosis of CVID, which are based on recent scientific discoveries. Improved diagnostic precision will assist with treatment decisions including IVIG/ scIG replacement. We suggest that asymptomatic patients with mild hypogammaglobulinaemia are termed hypogammaglobulinaemia of uncertain significance (HGUS). These patients require long-term follow-up, as some will evolve into CVID.

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Section: Discussionmentioning

confidence: 99%

Section: Proposed Definition Of Common Variable Immune Deficiency (mentioning

confidence: 99%

New diagnostic criteria for common variable immune deficiency (CVID), which may assist with decisions to treat with intravenous or subcutaneous immunoglobulin

Ameratunga

Woon

Gillis

et al. 2013

Clinical and Experimental Immunology

197

196

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“…In a retrospective study, patients with a selective or combined IgG subclass deficiency with four or more episodes of bacterial RTIs per year were treated with IVIg 0.4 mg/kg/month, which led to a 50% reduction in antibiotic-demanding (i.e. presumably bacterial) infections in 70% of patients (P < 0.001) [66].…”

Section: Page 9 Of 47mentioning

confidence: 99%

“…Patients with an IgG subclass deficiency or SADNI who suffer from severe or recurrent RTIs despite prophylactic antibiotics are candidates for Ig therapy [66,67].…”

Section: Indications For Igg Replacement Therapymentioning

confidence: 99%

Immunoglobulin treatment in primary antibody deficiency

Maarschalk-Ellerbroek

Hoepelman

Ellerbroek

2011

International Journal of Antimicrobial Agents

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The primary antibody deficiency syndromes are characterised by recurrent respiratory tract infections and the inability to produce effective immunoglobulin (Ig) responses. The best known primary antibody deficiencies are common variable immunodeficiency (CVID), X-linked agammaglobulinaemia (XLA), immunoglobulin G (IgG) subclass deficiency, and selective antibody deficiency with normal immunoglobulins (SADNI).Therapy in these patients consists of prophylactic antibiotics and/or Ig replacement therapy. Diagnostic delay remains common owing to limited awareness of the presenting features and may result in increased morbidity and mortality. Replacement therapy with immunoglobulins increases life expectancy and reduces the frequency and severity of infections, but the effect on end-organ damage is still unknown. Both intravenous immunoglobulin (IVIg) and subcutaneous immunoglobulin (SCIg) treatment appear to be safe, with comparable efficacy. A starting dose of 300-400 mg/kg/month in IVIg and 100 mg/week for SCIg is recommended. IgG trough levels should be >5 g/L for patients with agammaglobulinaemia and 3 g/L greater than the initial IgG level for patients with CVID; however, the clinical response should be foremost in choosing the dose and trough level. Infusion-related adverse reactions are generally mild owing to improved manufacturing processes. In this paper, aspects of Ig replacement therapy in primary antibody-deficient patients will be addressed.

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“…Similarly, administration of i.v. Igs is standard treatment, but evidence comes from cohort studies not from randomised controlled trials [203].…”

Section: Pharmacotherapy For Respiratory Tract Infections In Childrenmentioning

confidence: 99%

Respiratory medicines for children: current evidence, unlicensed use and research priorities

Smyth¹,

Barbato²,

Beydon³

et al. 2009

European Respiratory Journal

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This European Respiratory Society task force has reviewed the evidence for paediatric medicines in respiratory disease occurring in adults and children. We describe offlicence use, research priorities and ongoing studies.Off-licence and off-label prescribing in children is widespread and potentially harmful. Research areas in asthma include novel formulations and regimens, and individualised prescribing. In cystic fibrosis, future studies will focus on screened infants and robust outcome measures are needed. Other areas include new enzyme and antibiotic formulations and the basic defect. Research into pneumonia should include evaluation of new antibacterials and regimens, rapid diagnostic tests and, in pleural infection, antibiotic penetration, fibrinolytics and surveillance. In uncommon conditions, such as primary ciliary dyskinesia, congenital pulmonary abnormalities or neuromuscular disorders, drugs indicated for other conditions (e.g. dornase alfa) are commonly used and trials are needed. In neuromuscular disorders, the b-agonists may enhance muscle strength and are in need of evaluation. Studies of antibiotic prophylaxis, immunoglobulin and antifungal drugs are needed in immune deficiency.We hope that this summary of the evidence for respiratory medicines in children, highlighting gaps and research priorities, will be useful for the pharmaceutical industry, the paediatric committee of the European Medicines Agency, academic investigators and the lay public.

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Immunoglobulin prophylaxis in 350 adults with IgG subclass deficiency and recurrent respiratory tract infections: A long-term follow-up

Cited by 63 publications

References 16 publications

New diagnostic criteria for common variable immune deficiency (CVID), which may assist with decisions to treat with intravenous or subcutaneous immunoglobulin

New diagnostic criteria for common variable immune deficiency (CVID), which may assist with decisions to treat with intravenous or subcutaneous immunoglobulin

Immunoglobulin treatment in primary antibody deficiency

Respiratory medicines for children: current evidence, unlicensed use and research priorities

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